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Pediatric Rheumatology

, 9:P266 | Cite as

Juvenile systemic lupus erythematosus: a retrospective study in a single center over a 13 years period

  • N Conejo
  • O Porras
Open Access
Poster presentation

Keywords

Systemic Lupus Erythematosus Septic Shock Vasculitis Encephalitis Nephritis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Aim

To evaluate a group of children diagnosed as juvenile systemic lupus erythematosus (JSLE) in a single center over a 13-year period.

Methods

JSLE patients, age ≤ 18 years, were included. Clinical presentation, laboratory parameters and therapeutic approaches were analyzed at diagnosis. Outcome, response and adverse events to treatment were analyzed during the follow up period.

Results

74 cases were included, 78.3% females. The mean age at diagnosis was 9.9 years, 54% were children of age 6-10 years and 35.1% were of age 11-14 years. The most frequent pre-diagnosis manifestations were fever, arthralgias, asthenia, adinamia, weight loss, headaches and anorexia. The four most frequent classification criteria were a positive ANA (91.7%), positive immunoserology (95.9%), cytopenia (75.7%) and nephritis (58.1%). The main complications were associated to nephritis (58.1%), central nervous system (40.5%) and infection (24.3%). Seventy cases had flares, in relation with nephritis (35.7%), malar rash (27,1%), arthritis (20%), vasculitis (15.7%), serositis (11.4%) and cytopenias (10%). Fifteen children died (20.3%), due to septic shock, chronic renal insufficiency, autoimmune hepatitis, cerebral infarct, lung hemorrhage, hypertensive encephalitis, antiphospholipid syndrome and renal thrombosis. Death occurs a mean of 2.5 years (9 days to 7.9 years) after diagnosis.

Conclusions

A group of 74 children with JSLE were evaluated, most of them female. Half of the cases developed nephritis. A fifth of the cases died during the study period, mainly from infection. The analysis of the studied group allows us to obtain clinical information crucial to define diagnosis and treatment guidelines and develop medical education material.

Copyright information

© Conejo and Porras; licensee BioMed Central Ltd. 2011

This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Authors and Affiliations

  1. 1.Department of Pediatric Immunology and RheumatologyNational Children´s Hospital “Dr. Carlos Sáenz Herrera”San JoséCosta Rica

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